World sickle cell day is observed annually on the 19th of june to raise awareness of sickle cell-related diseases. It is observed to increase people's knowledge and the understanding required to face the challenges posed by the disease.


 

What is sickle cell disorder (scd)?

Sickle cell disorders or sickle cell diseases are genetic blood disorders that cause an abnormality in the red blood cells. The rbcs turn into sickle-shaped cells that stick together and block the blood flow in the body.


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Types

There are four major classifications of sickle cell diseases-


	
	Hemoglobin ss disease
	
	
	Hemoglobin sc disease
	
	
	Hemoglobin sb+ thalassemia
	
	
	Hemoglobin sb 0 thalassemia
	



 

Symptoms

One can start noticing symptoms of sickle cell in a patient at around 6 years of age. However, they can differ from person to person. Some of the most typical symptoms are-

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	Anemia
	


A shortage of red blood cells occurs due to the excessive early dying in 10-20 days. It leads to an oxygen shortage in the body.


	
	Excessive pain
	


Blockage of blood flow due to the change in shape leads to periodic cycles of pain in the body.


	
	Swelling in the body
	


As the blood circulation becomes irregular, swelling becomes a regular phenomenon.


	
	Infections
	


Sickle cell can be damaging to the spleen. It increases the risk of ailments in the body that can be prevented only by antibiotics.


	
	Delayed puberty
	


Oxygen and nutrients carried by the blood are required for growth. As a reason, irregular blood flow can lead to slow growth or delay in puberty.


	
	Vision issues
	


Infected blood vessels carrying blood to the eyes can cause damage to the retina. It can lead to visual problems.


 

Traits and treatment

As sickle cell is genetic, it is suggested to take a test that can detect any trait of the disease. It can prevent any life-threatening risks to the newborn. However, we don't have a complete cure for sickle cell disorders. There are preventive measures that reduce the adversity of the disease to an extent. Procedures like blood transfusions help greatly in minimizing the effects of the disorder.

World Sickle Cell Day: Know All About Sickle Cell Disease

C-reactive protein ;is a type of protein produced by the liver in response to an infection ;or inflammation. When your body contracts an infection, the white blood cells produce proteins to fight it. Some of these proteins activate the liver into producing c-reactive protein. For many years, the crp ;blood level has been used to ascertain the level of infection or inflammation.

Assessing the risk of heart disease ;with c-reactive protein

The level of crp is a potential indicator of how likely you are to develop cardiovascular diseases. This is because the development of atherosclerosis is linked with inflammation within the walls of your blood vessel. This results in higher levels of c-reactive protein in atherosclerosis patients than in those without the disorder.

The greater the crp levels, the more chances you have of being affected by cardiovascular disorders. The test also determines the risk of a second attack.

The value of knowing your crp levels depends on whether you come under one of three cardiovascular risk groups.


	
	High risk   not important to know crp because you should be combining lifestyle changes and aggressive treatment to reduce risk
	
	
	Intermediate risk   helpful to find out crp levels, as it can provide vital information crucial in deciding the next line of treatment
	
	
	Low risk   even if your crp levels are high, you will not be at risk   due to negligible risk factors and low cholesterol levels   that should require more than just lifestyle changes
	


To find out crp levels you will have to take a blood test. C-reactive protein is classified into  


	
	High   equal to or greater than 3
	
	
	Intermediate   between 1 and 3
	
	
	Low   below 1
	


If you are having a c-reactive protein test to check for heart diseases, your healthcare provider may recommend a cholesterol test as well. A number of other tests may also be prescribed to evaluate your risk further.

How C-reactive Protein Can Pose A Risk For Heart Disease?

What are the different types of sickle cell disease?

Sickle cell disease or sickle cell disorder is a term often used to talk about a group of diseases affecting hemoglobin. It is the oxygen-carrying element of blood that makes up 2 alpha globins and 2 beta globins. Sickle cell disease happens due to beta-globin mutation and depending on the abnormality in mutation.

Types-
Sickle cell diseases can be of many types depending on the gene mutation in the blood. Some of the common types are-

Sickle hemoglobin s disease (ss)
Hemoglobin ss disease or sickle cell anemia is the most popular type of scd. It takes place when hemoglobin s gene mutation is inherited from both mother and father. In simple words, it happens when the child inherits one substitution beta-globin gene from each parent. In this type, the body only produces hemoglobin s. It is mostly found in people of african and indian origin.

Sickle hemoglobin- c disease (sc):
Patients with sickle hemoglobin sc disease have a moderately dissimilar swapping in their genes of beta-globin. Here, both hemoglobin c and hemoglobin s are inherited from each parent. It may cause similar symptoms like anemia but remains relatively l. People of west african and mediterranean origin are more prone to this.

Hbs beta-thalassemia
People with this type of scd inherit one sickle cell gene s and one gene for beta-thalassemia from each parent. Zero and plus are the two common types of beta-thalassemia. While beta 0 thalassemia usually takes a severe form, beta + thalassemia is a milder form of the disease.

Sickle beta-zero thalassemia
Beta-zero thalassemia takes form when the child inherits the beta s gene from one parent and beta 0 thalassemia gene mutation from the other. With similar symptoms to anemia, it is a slightly severe form of beta-thalassemia.

Sickle beta-plus thalassemia
Substitutions in both beta-globin genes take place in patients with sickle beta thalassemia (sb) disease. This may even lead to the requirement of blood transfusion if no normal beta globin is produced. Mediterranean and caribbean people are more likely to have this disorder.

Hemoglobin sd, hemoglobin se, and hemoglobin so
These are the few rare and not less common types of sickle cell diseases. They are also less severe. People with these forms of scd inherit one sickle cell gene s and one gene from uncommon hemoglobin (d, e, or o) from each parent.

Sickle cell trait
Hbas
People with sct have one sickle cell gene s and one normal gene a from both mother and father. Ones with this disorder show few to no signs of the disease and usually spend a normal life. However, the risk to their future generations still exists.
 

What Are The Different Types Of Sickle Cell Disease?

Abstract

Background: it is essential to have an understanding of life expectancy and risk factors among patients with sickle cell disease (sickle cell anemia, hemoglobin diseases, and thalassemias). These are required to counsel the patients, target treatment, and plan the right clinical trials.

 

Methods: a research followed 3764 people ranging from birth to 66 years of age and suffering from scd. The procedure was done to understand the life expectancy and calculate the average age at death. Moreover, the study understood the reasons for the death in all 209 adults. It used the proportional-hazards regression analysis to know the risks for an untimely death in adults with sickle cell anemia.

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Results: when the results for young kids and adults with sickle cell anemia were observed, it was found that the average age at death was 48 for women and 42 for men. Moreover, suffering from hemoglobin c disease had an average mortality age of 60 for men and 68 years for women.

 

In people with sickle cell disease, 18% of deaths took place in patients with mainly renal organ failures. 33% of the tested patients were medically free of organ failures but episodes of pain crisis took their life. Out of these patients, 78% experienced chronic pain, chest syndrome, or both, while 22% suffered a stroke that took their lives.

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Clinical modeling showed that people with sickle cell anemia faced acute chest syndrome, seizures, kidney failure, a white-cell count of over 15, 000 per cubic mm, and a low hemoglobin level were related to a risk of early death.

 

Conclusions: results suggested that 50% of patients with sickle cell anemia lived beyond the age of 50. A huge share of those who lost their lives suffered no noticeable chronic organ failure. However, most of them died due to acute episodes of pain or stroke. Early death was seen highest among patients who had symptomatic disease. High levels of fetal hemoglobin suggested improved chances of survival and are assumably a credible source to foresee the risks and other factors related to adult life expectancy.


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Life Expectancy And Risk Factors Involved In Sickle Cell Disease

Preeclampsia is a condition that affects women during pregnancy. It is characterized by increased blood pressure and high levels of protein in the urine. Women with preeclampsia also experience swelling in the feet, legs, and arms.

Researchers suspect that excessive body fat or poor nutrition are the primary contributors to preeclampsia and eclampsia.

If left undetected, preeclampsia can lead to a more severe complication, known as eclampsia. Eclampsia can put you and your baby at risk, and can even lead to death (in rare cases).

How do preeclampsia and eclampsia affect the mother?

Preeclampsia and eclampsia can cause a host of complications for the mother, such as the following  

Hellp syndrome   hellp syndrome refers to three different conditions   low platelet count, elevated liver enzymes, and haemolysis. This generally occurs late in pregnancy and affects the processing of rbc, functioning of the liver, and blood clotting.

Seizures

Heart and renal failure

Water in the lungs

Bleeding from the liver

Reversible blindness

Effects of preeclampsia and eclampsia on the baby

Preeclampsia can lead to problems with the placenta in the initial stage of pregnancy. This may pose risks, including the following to the baby  

Lack of nutrients and oxygen, which may prevent the foetus to grow properly

Preterm birth

Stillbirth   this happens when the placenta is separated from the uterine wall

Infant death

Stillbirths are more common when the mother has suffered from a severe form of preeclampsia, like hellp syndrome.

Preeclampsia and eclampsia are linked to a myriad of health issues and life-long challenges for prematurely born babies. These may include cerebral palsy, learning disorders, epilepsy, deafness and blindness.

There is no particular cure for preeclampsia and eclampsia. Treatment, however, depends on how close you are to your due date, and how much the foetus has developed. In the case of mild preeclampsia, your doctor may recommend resting, healthy eating   that limits salt intake   and regular prenatal checkups.

Effects Of Preeclampsia And Eclampsia On You And The Baby

Type 1 diabetes occurs when your pancreas is unable to produce enough insulin to keep the blood sugar ;levels under control. The condition, if not managed properly, can lead to related health disorders and complications, both long-term and short-term.

Health problems arising from type 1 diabetes

Type 1 diabetes can have severe effects on your eyes, kidney, nervous system, and heart health. The most common ailments associated with type 1 diabetes may include the following-

1. Diabetic retinopathy

Diabetic retinopathy is a disease of the eyes, which occurs when the blood vessels in the retina (tissue present at the back of your eyes) are damaged. The walls of the retina gradually weaken from increasing blood pressure and blood sugar levels, and this leads to leakage of blood or fluid into the nearby tissues.

Early symptoms of diabetic retinopathy include-


	
	Blurred vision
	
	
	Floaters
	
	
	Difficulty perceiving colors
	


2. ;Diabetic neuropathy

Diabetic neuropathy unfolds when the nerves are damaged due to type 1 diabetes. The condition mostly affects your feet and legs. Reduced blood circulation due to high blood glucose impedes normal wound healing in the extremities, hence, even a minor bruise ;can develop into a permanent injury.

Symptoms of diabetic neuropathy are-


	
	Numbness
	
	
	Reduced sensation
	
	
	Severe, debilitating pain in the extremities
	


3. Diabetic nephropathy

Also known as ;diabetic kidney disease, the condition is a slow deterioration of the kidney and its ability to function properly. In severe cases, where the levels of blood glucose are extremely high due to type 1 diabetes, diabetic nephropathy can ultimately result in kidney failure ;or end-stage renal disease (esrd).

The following are the symptoms of diabetic nephropathy-


	
	Dark urine
	
	
	Swollen ankles, feet, eyes, or hands
	
	
	Shortness of breath
	


4. Cardiovascular diseases

Cardiovascular diseases refer to a range of heart conditions including blood clots and diseased blood vessels. High blood glucose levels resulting from type 1 diabetes can contribute to the deposition of fat in the walls of the blood vessel. Over time, this may restrict the flow of blood and put you at risk of cardiovascular disorders such as coronary artery disease, stroke, or even ;heart attack.

Symptoms of cardiovascular diseases widely vary, ranging from palpitations ;to chest pain. Sometimes, such a condition may not exhibit any symptoms at all.

Type 1 diabetes complications are related to poor management of blood glucose. The best way to monitor the condition and control the symptoms is to get in touch with your doctor. A specialist in this field can prescribe the correct treatment and help prevent the onset the related health disorders.

Complications Associated With Type 1 Diabetes

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